Unmasking catamenial hemoptysis in the era of CFTR modulator therapy

J Cyst Fibros. 2020 Jul;19(4):e25-e27. doi: 10.1016/j.jcf.2020.01.005. Epub 2020 Jan 24.

Abstract

Background: Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy.

Case summary: We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF).

Discussion: We review the literature on TES, pharmacologic management, and reported cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug interactions. We propose that our patient's recrudescence of catamenial hemoptysis was secondary to a drug-drug interaction between Lumacaftor/Ivacaftor and oral contraceptive therapy.

Conclusion: Our case suggests that women with CF who have catamenial hemoptysis and a genetic mutation approved for Tezacaftor/Ivacaftor or Elexacaftor/Tezacaftor/Ivacaftor can be managed effectively with either CFTR modulator and hormonal contraceptive therapy.

Keywords: CFTR modulators; Hemoptysis; Women's health.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aminophenols / therapeutic use*
  • Aminopyridines / therapeutic use*
  • Benzodioxoles / therapeutic use*
  • Bronchoscopy / methods
  • Chloride Channel Agonists / therapeutic use
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / drug therapy
  • Cystic Fibrosis* / physiopathology
  • Drug Combinations
  • Drug Therapy, Combination / methods
  • Endometriosis* / complications
  • Endometriosis* / diagnosis
  • Endometriosis* / drug therapy
  • Endometriosis* / physiopathology
  • Female
  • Fertility Agents, Female / administration & dosage
  • Hemoptysis* / diagnosis
  • Hemoptysis* / etiology
  • Hemoptysis* / prevention & control
  • Humans
  • Indoles / therapeutic use*
  • Leuprolide / administration & dosage*
  • Pyrazoles / therapeutic use*
  • Pyridines / therapeutic use*
  • Quinolines / therapeutic use*
  • Quinolones / therapeutic use*
  • Radiography, Thoracic / methods
  • Thoracic Diseases / complications
  • Thoracic Diseases / diagnosis
  • Thoracic Diseases / drug therapy
  • Thoracic Diseases / physiopathology
  • Tomography, X-Ray Computed / methods

Substances

  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • Chloride Channel Agonists
  • Drug Combinations
  • Fertility Agents, Female
  • Indoles
  • Pyrazoles
  • Pyridines
  • Quinolines
  • Quinolones
  • elexacaftor, ivacaftor, tezacaftor drug combination
  • lumacaftor, ivacaftor drug combination
  • Leuprolide